>> AT 26 WEEKS GESTATION, ADDISON HAD FETAL SURGERY
TO REMOVE WHAT WAS CALLED A "MEDIASTINAL TERATOMA,"
WHICH WAS A LARGE BENIGN MASS IN HER CHEST.
>> FIVE DAYS LATER, MARY STARTED TO HAVE SOME CONTRACTIONS.
ONE THING LED TO ANOTHER, AND THEY WEREN'T ABLE TO STOP
THE CONTRACTIONS, AND THEY HAD TO DELIVER THE BABY,
BOTH FOR THE HEALTH OF MARY AND FOR THE BABY.
>> AND SHE WAS RESUSCITATED RIGHT AWAY AT BIRTH AND PUT ON
LIFE SUPPORT AND TRANSFERRED TO THE NICU IMMEDIATELY.
>> AT THAT POINT THEY TOLD US THAT THEY DID NOT EXPECT ADDISON
TO MAKE IT, AND THAT WE SHOULD BE PREPARED FOR THAT.
AND WE PREPARED OURSELVES FOR THAT, BUT WE STILL PRAYED
AND HOPED THAT SHE WOULD.
AND THE NURSES AND THE DOCTORS IN THE NICU DID AN UNBELIEVABLE
JOB WITH ADDISON, AND WE WOULD LOOK FORWARD TO EACH NEW HURDLE
THAT SHE WOULD GET OVER.
AND ONE OF THE BIGGEST HURDLES THAT WAS ON THE HORIZON WAS
TO GET HER EXTUBATED.
>> THEY REMOVED THE BREATHING TUBE, AND RIGHT AWAY THEY COULD
TELL ADDISON WAS IN DISTRESS AND WAS NOT READY.
SO THEY IMMEDIATELY HAD TO REINTUBATE HER.
THEY SAID TO US, "SHE NEEDS THE VENTILATOR FOR THE FORESEEABLE
FUTURE, AND IN ORDER TO HELP HER GROW AND DEVELOP,
A TRACHEOSTOMY IS THE BEST WAY TO GIVE HER THE SUPPORT
OF THE VENTILATOR."
>> WHEN ADDISON GOT TO TRACHEOSTOMY TUBE,
IT CHANGED OUR PERSPECTIVE IMMEDIATELY ON WHAT
THAT REALLY MEANS.
YOUR AIRWAY IS ESSENTIAL TO YOUR SURVIVAL, AND YOU TAKE IT
FOR GRANTED UNTIL IT'S A SITUATION WHERE YOU CAN'T TAKE IT FOR GRANTED.
>> THERE'S A SAYING IN MEDICINE THAT THE AIRWAY COMES FIRST--
AIRWAY, BREATHING, AND CIRCULATION.
AND THAT'S SO IMPORTANT BECAUSE, IF YOU CAN'T BREATHE AND RESPIRE,
YOU REALLY CAN'T TAKE CARE OF THE OTHER SYSTEMS IN THE BODY.
>> THE AIRWAY SEEMS TO BE A VERY SIMPLE STRUCTURE.
IT'S ACTUALLY MULTIPLE COMPONENTS THAT WE TAKE
FOR GRANTED BECAUSE WE DON'T THINK ABOUT BREATHING.
>> WHEN A CHILD STRUGGLES TO BREATHE, IT'S A VERY FRIGHTENING
EXPERIENCE FOR THEM AND FOR THEIR FAMILIES TO WATCH IT HAPPEN.
>> ASIDE FROM THEIR UNDERLYING MEDICAL PROBLEM,
THERE ARE SO MANY OTHER ISSUES THAT AGAIN WE TAKE FOR GRANTED
THAT WE DO.
WE SPEAK.
WE BABBLE.
WE BREATHE.
WE COMMUNICATE.
WE FEED BY MOUTH.
WE EAT NORMALLY.
THESE CHILDREN DON'T.
>> IT IS AMAZING THE DEPTH OF COURAGE THAT THESE FAMILIES
HAVE IN DEALING WITH ALL THESE ISSUES.
>> I THINK THE ULTIMATE GOAL FOR MANY OF THESE FAMILIES
IS TO RESUME SOME SORT OF A NORMAL FAMILY LIFE,
SOMETHING THAT THEY HAVEN'T HAD FOR YEARS.
>> CHILDREN'S HOSPITAL OF PHILADELPHIA HAS SEVERAL AREAS
OF EXCELLENCE OR CENTERS THAT ARE NATIONALLY RENOWNED
FOR THE CARE THAT THEY GIVE.
AND THE PEDIATRIC AIRWAY PROGRAM HERE AT CHILDREN'S IS ONE
OF THOSE CENTERS.
>> WE CREATED THE CENTER FOR PEDIATRIC AIRWAY DISORDERS
TO SERVE CHILDREN WITH COMPLICATED AIRWAY DISORDERS,
AND THAT INCLUDES CHILDREN THAT HAVE TRACHEOTOMIES,
THAT HAVE NARROWING OF THE AIRWAY, OR HAVE CONGENITAL PROBLEMS
THAT THEY'RE BORN WITH, WHICH AFFECT THEIR AIR PASSAGES
AND BLOCK AND OBSTRUCT THE AIRWAY AND ARE VERY COMPLICATED
TO TAKE CARE OF.
>> WE THINK OF THE AIRWAY AS JUST OUR BREATHING,
BUT THE AIRWAY IS ACTUALLY A PRETTY COMPLEX ANATOMICAL
PATHWAY FROM THE NOSE INTO OUR TRACHEA.
>> AND THAT INCLUDES THE LARYNX OR SO-CALLED VOICE BOX,
THE TRACHEA, OR MORE COMMONLY CALLED "WINDPIPE,"
AND THE TWO BRANCHES OF THE WINDPIPE,
OR THE BRONCHI, WHICH GO TO THE LEFT AND RIGHT LUNG RESPECTIVELY.
AND WE FOCUS ON PROBLEMS THROUGHOUT THE AIRWAY.
>> AIRWAY DISORDERS IN BROAD TERMS CAN COMPRISE OF ISSUES
SUCH AS FLOPPINESS OF THE AIRWAY.
YOU CAN HAVE PARALYSIS WHERE THE ISSUE COULD BE RELATED
TO A NERVE NOT WORKING WELL.
OR YOU CAN HAVE A STENOSIS.
AND STENOSIS MEANS A NARROWING, WHICH CAN BE EITHER CONGENITAL,
SOMETHING YOU'RE BORN WITH, OR A STENOSIS THAT IS ACQUIRED.
>> ACQUIRED STENOSIS IS MOST COMMONLY CAUSED FROM PREMATURE
INFANTS WHO ARE ON A BREATHING MACHINE OR ON A VENTILATOR
FOR LONG PERIODS OF TIME.
THEY HAVE A FOREIGN BODY, OR AN ENDOTRACHEAL TUBE,
IN PLACE IN THE AIRWAY.
>> THE ENDOTRACHEAL TUBE CAN PUT SOME PRESSURE ON THE CARTILAGE
OF THE SUBGLOTTIS, AND THE LINING OF THE SUBGLOTTIS THEN
GETS IRRITATED, AND THE CRICOID CARTILAGE LOSES ITS BLOOD SUPPLY.
AND OVER TIME THAT INFLAMMATION CAN STIMULATE A REACTION
THAT CAN LEAD TO A SCARRING.
>> THE THIRD LESS COMMON CATEGORY OF STENOSIS WOULD BE TRAUMA.
TRAUMA FROM AUTOMOBILE ACCIDENTS, SPORTS INJURIES--
>> OR IT COULD BE A PENETRATING INJURY, SUCH AS A DOG BITE
OR STAB WOUNDS.
ANYTHING THAT ENTERS INTO THE CARTILAGE OF THE AIRWAY
CAN STIMULATE A REACTION THAT CAN LEAD TO SCARRING AND NARROWING.
>> WHEN ALEC TURNED 14, HE TRIED OUT FOR HIS FIRST TRAVEL
HOCKEY TEAM.
>> IT'S LIKE NORMAL PRACTICE.
WENT OUT ON THE ICE, AND WE WERE WARMING UP.
>> AND I MAYBE WAS GONE 10 MINUTES FROM DROPPING HIM OFF,
AND I GOT A PHONE CALL.
AND A WOMAN ON THE PHONE SAID THAT ALEC WAS STRUCK BY A PUCK.
>> I WAS SKATING BY THE NET AND ONE OF MY TEAMMATES TOOK A SLAP
SHOT, AND JUST IT WENT WIDE AND JUST HIT ME IN THE THROAT.
>> WE MET DR. JACOBS.
AND WHEN HE CAME IN, HE EXPLAINED TO US WHAT
WAS WRONG AND WHAT HIS PLAN WAS, AND HE FELT VERY CONFIDENT.
HE REALLY PUT US AT EASE THAT THEY WERE GOING TO BE ABLE
TO TAKE CARE OF HIM, TO TAKE CARE OF THE PROBLEM.
>> THERE'S A WIDE RANGE OF CHILDREN THAT WE CARE FOR IN THE AIRWAY--
IN THE PEDIATRIC AIRWAY PROGRAM.
AND THIS RANGES FROM BABIES WHO WERE JUST BORN TO TEENAGERS
WHO MAY BE 17 OR 18.
>> ONE OF THE BEAUTIES OF OUR PROGRAM IS THAT WE DON'T STOP
AT A CERTAIN AGE.
WE CAN STILL FOLLOW WITH A LOT OF THESE KIDS,
AND WE'RE COMFORTABLE WITH ALL OF THEM.